Case Presentation: A 78 year old woman with a PMH of asthma, sinusitis, CAD with stents and recent influenza presents with chest pain. Her EKG was without ST changes and troponin was 0.08 ng/mL. Other labs were significant for WBC of 10,900 cells/uL, and absolute eosinophil count 2800 cells/uL. She was diagnosed with NSTEMI and underwent LHC that was negative for obstructive disease and diagnosed with coronary artery vasospasm. She continued to have chest pain and her troponin rose to 12.5 ng/nL. A cardiac MRI was completed that was negative for signs of myocarditis but did show subendocardial late gadolinium enhancement (LGE). CT PE was negative for PE and revealed a LUL consolidation that was thought to be viral due to recent influenza and did not require antibiotics. Throughout this hospitalization her WBC increased to 11,400 cells/uL and eosinophils increased and peaked at 3000 cells/uL. Eosinophilia was presumed to be from previously known cough variant asthma or possible myeloproliferative disease. As her symptoms improved without treatment, she was discharged with hematology follow up. She returned to the hospital 10 days later with recurrent chest pain. Troponin was 1.44 ng/mL, WBC 10,900 cells/uL, eosinophils were 4,700 cells/uL, and creatinine increased from 0.6 mg/dL to 1.02 mg/dL with new onset hematuria and proteinuria. EKG did not demonstrate any significant changes and TTE showed slightly decreased EF to 50%. She was started on solumedrol for presumed hypereosinophilic syndrome with cardiac manifestations. She underwent a renal biopsy that was positive for necrotizing and crescentic glomerulonephritis and chronic interstitial inflammation with focal increased interstitial eosinophils.These findings with clinical history of eosinophilic asthma were consistent with eosinophilic granulomatosis with polyangiitis (EGPA). She was treated with prednisone and cyclophosphamide infusion over a 15 week period with improvement.
Discussion: EGPA (formerly Churg-Strauss syndrome) is a small to medium vessel autoimmune vasculitis associated with eosinophilic asthma, positive anti-myeloperoxidase and positive ANCA. It typically develops in three phases 1) The allergic phase with development of asthma, allergic rhinitis and sinusitis; 2) The eosinophilic phase with eosinophilic organ involvement including lung, heart, GI and renal system; and 3) vasculitic phase characterized by purpura, peripheral neuropathy and constitutional symptoms. Cardiac involvement affects 60% of patients. It can present in a variety of ways and can involve the endocardium, myocardium, pericardium, and coronary vessels sometimes causing heart failure and cardiogenic shock. Cardiac MRI with LGE is consistent with fibrosis and inflammation of the myocardium that is usually caused by vasculitis of the small myocardial vasculature. Cardiac involvement is a poor prognostic indicator in EGPA and early detection and treatment are crucial to prevent cardiac disease progression.
Conclusions: A patient with undiagnosed EGPA can present with a variety of symptoms, including those mimicking myocardial infarction. In patients with an elevated troponin with eosinophilia, asthma and sinusitis without evidence of coronary artery disease, EGPA should be considered. Since cardiac involvement is a poor prognostic indicator, early detection and treatment is important to halt disease progression.